Journal of Postgraduate Gynecology & Obstetrics is an Open Access, peer reviewed online journal published by Department of Obstetrics and Gynecology of Seth G. S. Medical College and K.E.M. Hospital, Parel, Mumbai, 400012, India.

Takayasu
arteritis (TKA) is a vasculitic disorder characterized by involvement
of aortic arch and its branches. In pregnant patients, it is commonly
associated with preeclampsia, intrauterine fetal demise (IUFD) and
increased morbidity. A multigravid patient presenting with severe
pre-eclampsia and IUFD, who was found to have Takayasu arteritis is
reported here.

Introduction

Preeclampsia
presents in myriad ways. In a patient who presents in the index
pregnancy with severe preeclampsia with no prior medical evaluation,
a thorough initial examination is imperative, and can lead to
detection of hereto unrecognized systemic disturbances.

Case
Report

A
30-year-old female married since 17 years G4P2L2A1, with eight months
gestation presented to our emergency room with chief complaints of
abdominal pain, giddiness and vomiting. She was unregistered,
non-immunized, and was apparently asymptomatic till the day of
presentation. Headache started in the occipital region and was
throbbing in nature. There were no complaints of vaginal bleeding,
convulsions or decreased urine output. There was no previous
significant major medical or surgical history. She had two normal,
uneventful home deliveries.

Patient
was conscious and well oriented, with a pulse of 88 beats per minute,
recorded in right radial artery and blood pressure of 170/110 mm Hg
recorded in the right brachial artery. Pulses in left radial and
brachial artery were not palpable, while other peripheral pulsations
on left side, namely femoral, and dorsalis pedis were palpable. There
were no ischemic changes over the left upper limb. History was
reviewed and patient did not complain of any pain, claudication or
weakness of the left upper limb. Neurological assessment did not
reveal any abnormality. A physician was consulted and Doppler
studies were advised. Cardiovascular and respiratory systems were
unremarkable. On abdominal examination uterus was relaxed, 26 weeks
size, and fetus in vertex presentation. Fetal heart sounds could not
be heard on Doppler, and ultrasonography confirmed IUFD. Internal
cervical os was closed and uneffaced. Premonitory symptoms were
present in the form of headache, vomiting and epigastric pain, hence
magnesium sulfate by Pritchard regimen was started.

A
few hours later, patient developed a syncopal attack, from which she
improved within a few minutes. Magnesium sulfate was discontinued and
injectable phenytoin was started after physician consultation.
Through the course of her stay in the hospital for around 6 days, she
developed 4 episodes of syncope. History was further reviewed and
patient reported having similar episodes of syncope occasionally,
which had never been reported or evaluated before.

Investigations
revealed mild thrombocytopenia, elevated liver enzymes (SGOT 244 and
SGPT 178U/l) and otherwise normal hematological and biochemical
profile. Pre-induction ripening of cervix was done with Foley
catheter. Labor was augmented with oxytocin and a fresh still born
female of 1024 grams was delivered. Lactation suppression was
initiated.

Upper
limb Doppler flows were studied which showed monophasic waveform in
left ulnar, radial, brachial arteries, suggestive of occlusion. CT
angiogram done post delivery confirmed long segmental complete
occlusion of left subclavian and left axillary artery, with presence
of calcification. However, there was no evidence of thrombosis.
Cardiac echogram showed structurally normal heart without any
valvular lesions or pulmonary artery hypertension. Doppler of both
renal arteries did not show any evidence of renal artery stenosis.

Serum
TSH was high (9.14) hence l 50 microgram of levothyroxine was
started. Autoimmune markers were sent, but were negative for ANA,
Anti-dsDNA, LA, aCL antibodies (IgM & IgG). Further evaluation,
steroids and stenting of the subclavian artery will be planned on
follow up after the puerperal period.

Figure 1.
CT angiography (arrow pointing to subclavian stenosis) .

Discussion

The
initial presentation of the patient was similar to classic
presentation of severe preeclampsia, except that the asymmetric pulse
and blood pressure recording pointed towards a peripheral arterial
problem. However, the diagnosis of Takayasu arteritis could be
ascertained only after the results of the imaging features was
suggestive of subclavian stenosis. The occurrence of syncopal attacks
also strongly supported the diagnosis. Ours being a tertiary academic
center, such diagnosis could be reached with multi-disciplinary
consultation.

Takayasu's
arteritis is a chronic, relapsing, stenotic, inflammatory disease of
medium and large-sized arteries with a propensity for the aortic arch
and its branches. Incidence is around 2.5 cases per million and is
more prevalent among young women and in Asians.

Hypertension
is seen in up to 90% of patients with TKA.[1] However, since the
patient had not received any prior antenatal care, it is postulated
that hypertension was present but not detected due to lack of medical
attention; her presentation was one of hypertension with superimposed
preeclampsia.

Though
vasculitis, i.e. histopathologic demonstration of inflamed vessels is
the gold standard, it is rarely required for establishing the
diagnosis, and was not done in our patient.[1] The classical CT
angiographic findings include transmural calcific changes and luminal
narrowing,[2] both of which were present in our patient (Figure 1).
Narrowing of the arterial lumen occurs with thrombosis; however CT
scan in our patient did not show the presence of any thrombus.

Pregnancies
with Takayasu arteritis have been reported to have a 13-fold higher
rate of obstetric complications compared to normal pregnancies. [3]
Our patient presented with a blood pressure of 170/110 mmHg and
intrauterine fetal demise. Pre-eclampsia, premature delivery and IUFD
have been reported to be of higher incidence in patients with TKA.[4,
5, 6]

There
were no cardiac events or electrocardiographic abnormalities in our
patient. Cardiovascular events are a common feature in this
condition. However the occurrence of the same during pregnancy has
been reported to be rare,[7] as was in our case. Pulmonary
hypertension is a common feature,[8] but it was ruled out in our case
on cardiac echogram. The occurrence of renal artery stenosis along
with concomitant subclavian stenosis detected in pregnancy has also
been reported by Nalini et al.[9]

Our
patient was also evaluated objectively for antiphospholipid antibody
syndrome (APLA). ANA, Anti-ds DNA, aCL, LA, which were all negative.
However, Anti-beta 2 glycoprotein antibody could not be done due to
financial constraints. Hence, concomitant APLA syndrome cannot be
ruled out. It is interesting to note that aortoarteritis has been
postulated to be a precursor to APLA syndrome.[10, 11]

The
treatment principles include glucocorticoids, control of
hypertension, prevention of renal failure, and arterioplastic
approach to stenosed vessels.[1]

To
conclude, a vigilant initial general examination led to an early
suspicion of a concomitant systemic cause in this patient, a rare
vasculitic syndrome. Like in this case, grave medical disorders could
first be recognized during pregnancy, and the opportunity should be
used for thorough investigation and management. Control of blood
pressure appears to be the cornerstone in the management of this
disease in pregnancy. A multidisciplinary approach is needed to
ensure good obstetric and neonatal outcome. This case is being
presented due the novel occurrence of the rare vasculitic condition
in pregnancy.